Harrison's Cardiovascular Medicine 2 ed.


Eugene Braunwald


The normal pericardium is a double-layered sac; the visceral pericardium is a serous membrane that is separated by a small quantity (15–50 mL) of fluid, an ultra-filtrate of plasma, from the fibrous parietal pericardium. The normal pericardium, by exerting a restraining force, prevents sudden dilation of the cardiac chambers, especially the right atrium and ventricle, during exercise and with hypervolemia. It also restricts the anatomic position of the heart, minimizes friction between the heart and surrounding structures, prevents displacement of the heart and kinking of the great vessels, and probably retards the spread of infections from the lungs and pleural cavities to the heart. Nevertheless, total absence of the pericardium, either congenital or after surgery, does not produce obvious clinical disease. In partial left pericardial defects, the main pulmonary artery and left atrium may bulge through the defect; very rarely, herniation and subsequent strangulation of the left atrium may cause sudden death.


Acute pericarditis, by far the most common pathologic process involving the pericardium, may be classified both clinically and etiologically (Table 22-1). There are four principal diagnostic features:

TABLE 22-1




1. Chest pain is an important but not invariable symptom in various forms of acute pericarditis (Chap. 4); it is usually present in the acute infectious types and in many of the forms presumed to be related to hypersensitivity or autoimmunity. Pain is often absent in slowly developing tuberculous, post-irradiation, neoplastic, and uremic pericarditis. The pain of acute pericarditis is often severe, retrosternal and left precordial, and referred to the neck, arms, or left shoulder. Often the pain is pleuritic, consequent to accompanying pleural inflammation (i.e., sharp and aggravated by inspiration and coughing), but sometimes it is a steady, constricting pain that radiates into either arm or both arms and resembles that of myocardial ischemia; therefore, confusion with acute myocardial infarction (AMI) is common. Characteristically, however, pericardial pain may be relieved by sitting up and leaning forward and is intensified by lying supine. The differentiation of AMI from acute pericarditis becomes perplexing when, with acute pericarditis, serum biomarkers of myocardial damage such as creatine kinase and troponin rise, presumably because of concomitant involvement of the epicardium in the inflammatory process (an epi-myocarditis) with resulting myocyte necrosis. However, these elevations, if they occur, are quite modest given the extensive electrocardiographic ST-segment elevation in pericarditis. This dissociation is useful in differentiating between these conditions.

2. A pericardial friction rub is audible in about 85% of these patients, may have up to three components per cardiac cycle, is high pitched, and is described as rasping, scratching, or grating (Chap. 9); it can be elicited sometimes when the diaphragm of the stethoscope is applied firmly to the chest wall at the left lower sternal border. It is heard most frequently at end expiration with the patient upright and leaning forward. The rub is often inconstant, and the loud to-and-fro leathery sound may disappear within a few hours, possibly to reappear on the next day. A pericardial rub is heard throughout the respiratory cycle, whereas a pleural rub disappears when respiration is suspended.

3. The electrocardiogram (ECG) in acute pericarditis without massive effusion usually displays changes secondary to acute subepicardial inflammation (Fig. 22-1). It typically evolves through four stages. In stage 1, there is widespread elevation of the ST segments, often with upward concavity, involving two or three standard limb leads and V2 to V6, with reciprocal depressions only in aVR and sometimes V1, as well as depression of the PR segment below the TP segment reflecting atrial involvement. Usually, there are no significant changes in QRS complexes. In stage 2, after several days, the ST segments return to normal, and only then, or even later, do the T waves become inverted (stage 3). Ultimately, weeks or months after the onset of acute pericarditis, the ECG returns to normal in stage 4. In contrast, in AMI, ST elevations are convex, and reciprocal depression is usually more prominent; QRS changes occur, particularly the development of Q waves, as well as notching and loss of R-wave amplitude, and T-wave inversions are usually seen within hours before the ST segments have become isoelectric. Sequential ECGs are useful in distinguishing acute pericarditis from AMI. In the latter, elevated ST segments return to normal within hours (Chaps. 34 and 35).



Acute pericarditis often produces diffuse ST-segment elevations
 (in this case in leads I, II, aVF, and V2 to V6) due to a ventricular current of injury. Note also the characteristic PR-segment deviation (opposite in polarity to the ST segment) due to a concomitant atrial injury current.

Early repolarization is a normal variant and may also be associated with widespread ST-segment elevation, most prominent in left precordial leads. However, in this condition the T waves are usually tall and the ST/T ratio is <0.25; importantly, this ratio is higher in acute pericarditis.

4. Pericardial effusion is usually associated with pain and/or the ECG changes mentioned earlier, as well as with an enlargement of the cardiac silhouette. Pericardial effusion is especially important clinically when it develops within a relatively short time as it may lead to cardiac tamponade (see later). Differentiation from cardiac enlargement may be difficult on physical examination, but heart sounds may be fainter with pericardial effusion. The friction rub may disappear, and the apex impulse may vanish, but sometimes it remains palpable, albeit medial to the left border of cardiac dullness. The base of the left lung may be compressed by pericardial fluid, producing Ewart’s sign, a patch of dullness and increased fremitus (and egophony) beneath the angle of the left scapula. The chest roentgenogram may show a “water bottle” configuration of the cardiac silhouette (Fig. 22-2) but may be normal.



Chest radiogram
 from a patient with a pericardial effusion showing typical “water bottle” heart. There is also a right pleural effusion. (From SS Kabbani, M LeWinter, in MH Crawford et al [eds]: Cardiology. London, Mosby, 2001.)


Echocardiography (Chap. 12) is the most widely used imaging technique since it is sensitive, specific, simple, and noninvasive; may be performed at the bedside; and can identify accompanying cardiac tamponade (see later) (Fig. 22-3). The presence of pericardial fluid is recorded by two-dimensional transthoracic echocardiography as a relatively echo-free space between the posterior pericardium and left ventricular epicardium in patients with small effusions and as a space between the anterior right ventricle and the parietal pericardium just beneath the anterior chest wall in those with larger effusions. In the latter, the heart may swing freely within the pericardial sac. When severe, the extent of this motion alternates and may be associated with electrical alternans. Echocardiography allows localization and estimation of the quantity of pericardial fluid.



Apical four-chamber echocardiogram
 recorded in a patient with a moderate pericardial effusion and evidence of hemodynamic compromise. The frame is recorded in early ventricular systole, immediately after atrial contraction. Note that the right atrial wall is indented inward and its curvature is frankly reversed (arrow), implying elevated intrapericardial pressure above right atrial pressure. LA, left atrium; LV, left ventricle; RV, right ventricle. (From WF Armstrong: Echocardiography, in DP Zipes et al [eds]: Braunwald’s Heart Disease, 7th ed. Philadelphia, Elsevier, 2005.)

The diagnosis of pericardial fluid or thickening may be confirmed by computed tomography (CT) or magnetic resonance imaging (MRI) (Fig. 22-4). These techniques may be superior to echocardiography in detecting loculated pericardial effusions, pericardial thickening, and the presence of pericardial masses.



Chronic pericardial effusion
 in a 54-year-old female patient with Hodgkin’s disease seen in contrast-enhanced 64-slice CT. The arrows point at the pericardial effusion (LV, left ventricle; RV, right ventricle; RA, right atrium). Due to the timing of the scan relative to contrast injection, only the blood in the left ventricle is contrast-enhanced, hence, the low attenuation in the right-sided chambers. (From Achenbach S, Daniel WG: Computed Tomography of the Heart, in P Libby et al [eds]: Braunwald’s Heart Disease, 8th ed. Philadelphia, Elsevier, 2008.)


The accumulation of fluid in the pericardial space in a quantity sufficient to cause serious obstruction to the inflow of blood to the ventricles results in cardiac tamponade. This complication may be fatal if it is not recognized and treated promptly. The three most common causes of tamponade are neoplastic disease, idiopathic pericarditis, and renal failure. Tamponade may also result from bleeding into the pericardial space after cardiac operations, trauma, and treatment of patients with acute pericarditis with anticoagulants.

The three principal features of tamponade (Beck’s triad) are hypotension, soft or absent heart sounds, and jugular venous distention with a prominent x descent but an absent y descent. There are both limitation of ventricular filling and reduction of cardiac output. The quantity of fluid necessary to produce this critical state may be as small as 200 mL when the fluid develops rapidly or >2000 mL in slowly developing effusions when the pericardium has had the opportunity to stretch and adapt to an increasing volume. Tamponade may also develop more slowly, and in these circumstances the clinical manifestations may resemble those of heart failure, including dyspnea, orthopnea, and hepatic engorgement. A high index of suspicion for cardiac tamponade is required since in many instances no obvious cause for pericardial disease is apparent, and it should be considered in any patient with otherwise unexplained enlargement of the cardiac silhouette, hypotension, and elevation of jugular venous pressure. There may be reduction in amplitude of the QRS complexes, and electrical alternans of the P, QRS, or T waves should raise the suspicion of cardiac tamponade.

Table 22-2 lists the features that distinguish acute cardiac tamponade from constrictive pericarditis.

TABLE 22-2



Paradoxical pulse

This important clue to the presence of cardiac tamponade consists of a greater than normal (10 mmHg) inspiratory decline in systolic arterial pressure. When severe, it may be detected by palpating weakness or disappearance of the arterial pulse during inspiration, but usually sphygmomanometric measurement of systolic pressure during slow respiration is required.

Since both ventricles share a tight incompressible covering, i.e., the pericardial sac, the inspiratory enlargement of the right ventricle in cardiac tamponade compresses and reduces left ventricular volume; leftward bulging of the interventricular septum further reduces the left ventricular cavity as the right ventricle enlarges during inspiration. Thus, in cardiac tamponade the normal inspiratory augmentation of right ventricular volume causes an exaggerated reciprocal reduction in left ventricular volume. Also, respiratory distress increases the fluctuations in intrathoracic pressure, which exaggerates the mechanism just described. Right ventricular infarction (Chap. 35) may resemble cardiac tamponade with hypotension, elevated jugular venous pressure, an absent y descent in the jugular venous pulse, and, occasionally, pulsus paradoxus. The differences between these two conditions are shown in Table 22-2.

Paradoxical pulse occurs not only in cardiac tamponade but also in approximately one-third of patients with constrictive pericarditis (see later). This physical finding is not pathognomonic of pericardial disease because it may be observed in some cases of hypovolemic shock, acute and chronic obstructive airway disease, and pulmonary embolus.

Low-pressure tamponade refers to mild tamponade in which the intrapericardial pressure is increased from its slightly subatmospheric levels to +5 to +10 mmHg; in some instances, hypovolemia coexists. As a consequence, the central venous pressure is normal or only slightly elevated, whereas arterial pressure is unaffected and there is no paradoxical pulse. These patients are asymptomatic or complain of mild weakness and dyspnea. The diagnosis is aided by echocardiography, and both hemodynamic and clinical manifestations improve after pericardiocentesis.


Since immediate treatment of cardiac tamponade may be lifesaving, prompt measures to establish the diagnosis by echocardiography should be undertaken (Fig. 22-3). When pericardial effusion causes tamponade, Doppler ultrasound shows that tricuspid and pulmonic valve flow velocities increase markedly during inspiration, whereas pulmonic vein, mitral, and aortic flow velocities diminish. Often the right ventricular cavity is reduced in diameter, and there is late diastolic inward motion (collapse) of the right ventricular free wall and the right atrium. Transesophageal echocardiography may be necessary to diagnose a loculated or hemorrhagic effusion responsible for cardiac tamponade.

TREATMENT Cardiac Tamponade

Patients with acute pericarditis should be observed frequently for the development of an effusion; if a large effusion is present, the patient should be hospitalized and pericardiocentesis carried out or the patient should be watched closely for signs of tamponade. Arterial and venous pressures and heart rate should be monitored or followed carefully, and serial echocardiograms obtained.

PERICARDIOCENTESIS If manifestations of tamponade appear, echocardiographically or fluoroscopically guided pericardiocentesis using an apical, parasternal, or, most commonly, subxiphoid approach must be carried out at once as reduction of the elevated intrapericardial pressure may be lifesaving. Intravenous saline may be administered as the patient is being readied for the procedure, but the pericardiocentesis must not be delayed. If possible, intrapericardial pressure should be measured before fluid is withdrawn, and the pericardial cavity should be drained as completely as possible. A small, multiholed catheter advanced over the needle inserted into the pericardial cavity may be left in place to allow draining of the pericardial space if fluid reaccumulates. Surgical drainage through a limited (subxiphoid) thoracotomy may be required in recurrent tamponade, when it is necessary to remove loculated effusions, and/or when it is necessary to obtain tissue for diagnosis.

Pericardial fluid obtained from an effusion often has the physical characteristics of an exudate. Bloody fluid is most commonly due to neoplasm in the United States and tuberculosis in developing nations but may also be found in the effusion of acute rheumatic fever, post-cardiac injury, and postmyocardial infarction, as well as in the pericarditis associated with renal failure or dialysis. Transudative pericardial effusions may occur in heart failure.

The pericardial fluid should be analyzed for red and white blood cells, and cytologic studies for cancer, microscopic studies, and cultures should be obtained. The presence of DNA of Mycobacterium tuberculosis determined by polymerase chain reaction or an elevated adenosine deaminase activity (>30 U/L) strongly supports the diagnosis of tuberculous pericarditis.


In many instances, acute pericarditis occurs in association with illnesses of known or presumed viral origin and probably is caused by the same agent. Commonly, there is an antecedent infection of the respiratory tract, and viral isolation and serologic studies are negative. In some cases, coxsackievirus A or B or the virus of influenza, echovirus, mumps, herpes simplex, chickenpox, adenovirus, cytomegalovirus, Epstein-Barr, or HIV has been isolated from pericardial fluid and/or appropriate elevations in viral antibody titers have been noted. Pericardial effusion is a common cardiac manifestation of HIV; it is usually secondary to infection (often myco-bacterial) or neoplasm, most frequently lymphoma. Most frequently, a viral causation cannot be established; the term idiopathic acute pericarditis is then appropriate. Viral or idiopathic acute pericarditis occurs at all ages but is more common in young adults and is often associated with pleural effusions and pneumonitis. The almost simultaneous development of fever and precordial pain, often 10 to 12 days after a presumed viral illness, constitutes an important feature in the differentiation of acute pericarditis from AMI, in which chest pain precedes fever. The constitutional symptoms are usually mild to moderate, and a pericardial friction rub is often audible. The disease ordinarily runs its course in a few days to 4 weeks. The ST-segment alterations in the ECG usually disappear after 1 or more weeks, but the abnormal T waves may persist for several years and be a source of confusion in persons without a clear history of pericarditis.

Pleuritis and pneumonitis frequently accompany pericarditis. Accumulation of some pericardial fluid is common, and both tamponade and constrictive pericarditis are possible complications. Recurrent (relapsing) pericarditis occurs in about one-fourth of patients with acute idiopathic pericarditis. In a smaller number, there are multiple recurrences.

TREATMENT Idiopathic Acute Pericarditis

In acute idiopathic pericarditis there is no specific therapy, but bed rest and anti-inflammatory treatment with aspirin (2–4 g/d) may be given. If this is ineffective, one of the nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (400–600 mg tid), indomethacin (25–50 mg tid), or colchicine (0.6 mg bid), is often effective. Glucocorticoids (e.g., prednisone, 40–80 mg daily) usually suppress the clinical manifestations of the acute illness and may be useful in patients in whom purulent bacterial pericarditis has been excluded and in patients with pericarditis secondary to connective tissue disorders and renal failure (see later). Anticoagulants should be avoided since their use could cause bleeding into the pericardial cavity and tamponade.

After the patient has been asymptomatic and afebrile for about a week, the dose of the NSAID may be tapered gradually. Colchicine may prevent recurrences, but when recurrences are multiple, frequent, and disabling; continued beyond 2 years; and are not controlled by glucocorticoids, pericardiectomy may be necessary to terminate the illness.

Postcardiac injury syndrome

Acute pericarditis may appear in a variety of circumstances that have one common feature: previous injury to the myocardium with blood in the pericardial cavity. The syndrome may develop after a cardiac operation (postpericardiotomy syndrome), after blunt or penetrating cardiac trauma (Chap. 23), or after perforation of the heart with a catheter. Rarely, it follows AMI.

The clinical picture mimics acute viral or idiopathic pericarditis. The principal symptom is the pain of acute pericarditis, which usually develops 1 to 4 weeks after the cardiac injury (1 to 3 days after AMI) but sometimes appears only after an interval of months. Recurrences are common and may occur up to 2 years or more after the injury. Pericarditis, fever with temperature up to 39°C (102.2°F), pleuritis, and pneumonitis are the outstanding features, and the bout of illness usually subsides in 1 or 2 weeks. The pericarditis may be of the fibrinous variety, or it may be a pericardial effusion, which is often serosanguineous but rarely causes tamponade. Leukocytosis, an increased sedimentation rate, and ECG changes typical of acute pericarditis may also occur.

This syndrome is probably the result of a hypersensitivity reaction to antigen that originates from injured myocardial tissue and/or pericardium. Circulating myocardial antisarcolemmal and antifibrillar autoantibodies occur frequently, but their precise role in the development of this syndrome has not been defined. Viral infection may also play an etiologic role, since antiviral antibodies are often elevated in patients who develop this syndrome after cardiac surgery.

Often no treatment is necessary aside from aspirin and analgesics. When the illness is followed by a series of disabling recurrences, therapy with an NSAID, colchicine, or a glucocorticoid is usually effective.


Since there is no specific test for acute idiopathic pericarditis, the diagnosis is one of exclusion. Consequently, all other disorders that may be associated with acute fibrinous pericarditis must be considered. A common diagnostic error is mistaking acute viral or idiopathic pericarditis for AMI and vice versa. When acute fibrinous pericarditis is associated with AMI (Chap. 35), it is characterized by fever, pain, and a friction rub in the first 4 days after the development of the infarct. ECG abnormalities (such as the appearance of Q waves, brief ST-segment elevations with reciprocal changes, and earlier T-wave changes in AMI) and the extent of the elevations of myocardial enzymes are helpful in differentiating pericarditis from AMI.

Pericarditis secondary to postcardiac injury is differentiated from acute idiopathic pericarditis chiefly by timing. If it occurs within a few days or weeks of an AMI, a chest blow, a cardiac perforation, or a cardiac operation, it may be justified to conclude that the two are probably related.

It is important to distinguish pericarditis due to collagen vascular disease from acute idiopathic pericarditis. Most important in the differential diagnosis is the pericarditis due to systemic lupus erythematosus (SLE) or drug-induced (procainamide or hydralazine) lupus. When pericarditis occurs in the absence of any obvious underlying disorder, the diagnosis of SLE may be suggested by a rise in the titer of antinuclear antibodies. Acute pericarditis is an occasional complication of rheumatoid arthritisscleroderma, and polyarteritis nodosa, and other evidence of these diseases is usually obvious. Asymptomatic pericardial effusion is also common in these disorders. The pericarditis of acute rheumatic fever is generally associated with evidence of severe pancarditis and with cardiac murmurs (Chap. 26).

Pyogenic (purulent) pericarditis is usually secondary to cardiothoracic operations, by extension of infection from the lungs or pleural cavities, from rupture of the esophagus into the pericardial sac, or from rupture of a ring abscess in a patient with infective endocarditis, or it can occur if septicemia complicates aseptic pericarditis. It is usually accompanied by fever, chills, septicemia, and evidence of infection elsewhere and generally has a poor prognosis. The diagnosis is made by examination of the pericardial fluid. Acute pericarditis may also complicate the viral, pyogenic, mycobacterial, and fungal infections that occur with HIV infection.

Pericarditis of renal failure occurs in up to one-third of patients with chronic uremia (uremic pericarditis), is also seen in patients undergoing chronic dialysis with normal levels of blood urea and creatinine, and is termed dialysis-associated pericarditis. These two forms of pericarditis may be fibrinous and are generally associated with an effusion that may be sanguineous. A pericardial friction rub is common, but pain is usually absent or mild. Treatment with an NSAID and intensification of dialysis are usually adequate. Occasionally, tamponade occurs and pericardiocentesis is required. When the pericarditis of renal failure is recurrent or persistent, a pericardial window should be created or pericardiectomy may be necessary.

Pericarditis due to neoplastic diseases results from extension or invasion of metastatic tumors (most commonly carcinoma of the lung and breast, malignant melanoma, lymphoma, and leukemia) to the pericardium; pain, atrial arrhythmias, and tamponade are complications that occur occasionally. Diagnosis is made by pericardial fluid cytology or pericardial biopsy. Mediastinal irradiation for neoplasm may cause acute pericarditis and/or chronic constrictive pericarditis. Unusual causes of acute pericarditis include syphilis, fungal infection (histoplasmosis, blastomycosis, aspergillosis, and candidiasis), and parasitic infestation (amebiasis, toxoplasmosis, echinococcosis, trichinosis).


Chronic pericardial effusions are sometimes encountered in patients without an antecedent history of acute pericarditis. They may cause few symptoms per se, and their presence may be detected by finding an enlarged cardiac silhouette on chest roentgenogram. Tuberculosis is a common cause.

Other causes

Myxedema may be responsible for chronic pericardial effusion that is sometimes massive but rarely, if ever, causes cardiac tamponade. The cardiac silhouette is markedly enlarged, and an echocardiogram distinguishes cardiomegaly from pericardial effusion. The diagnosis of myxedema can be confirmed by tests for thyroid function. Myxedematous pericardial effusion responds to thyroid hormone replacement.

Neoplasms, systemic lupus erythematosus (SLE), rheumatoid arthritis, mycotic infections, radiation therapy to the chest, pyogenic infections, and chylopericardium may also cause chronic pericardial effusion and should be considered and specifically sought in such patients.

Aspiration and analysis of the pericardial fluid are often helpful in diagnosis. Pericardial fluid should be analyzed as described earlier. Grossly sanguineous pericardial fluid results most commonly from a neoplasm, tuberculosis, renal failure, or slow leakage from an aortic aneurysm. Pericardiocentesis may resolve large effusions, but pericardiectomy may be required with recurrence. Intrapericardial instillation of sclerosing agents or antineoplastic agents may be used to prevent reaccumulation of fluid.


This disorder results when the healing of an acute fibrinous or serofibrinous pericarditis or the resorption of a chronic pericardial effusion is followed by obliteration of the pericardial cavity with the formation of granulation tissue. The latter gradually contracts and forms a firm scar, which may be calcified, encasing the heart and interfering with filling of the ventricles. In developing nations where the condition is prevalent, a high percentage of cases are of tuberculous origin, but this is now an uncommon cause in North America. Chronic constrictive pericarditis may follow acute or relapsing viral or idiopathic pericarditis, trauma with organized blood clot, cardiac surgery of any type, mediastinal irradiation, purulent infection, histoplasmosis, neoplastic disease (especially breast cancer, lung cancer, and lymphoma), rheumatoid arthritis, SLE, and chronic renal failure with uremia treated by chronic dialysis. In many patients the cause of the pericardial disease is undetermined, and in them an asymptomatic or forgotten bout of viral pericarditis, acute or idiopathic, may have been the inciting event.

The basic physiologic abnormality in patients with chronic constrictive pericarditis is the inability of the ventricles to fill because of the limitations imposed by the rigid, thickened pericardium. In constrictive pericarditis, ventricular filling is unimpeded during early diastole but is reduced abruptly when the elastic limit of the pericardium is reached, whereas in cardiac tamponade, ventricular filling is impeded throughout diastole. In both conditions, ventricular end-diastolic and stroke volumes are reduced and the end-diastolic pressures in both ventricles and the mean pressures in the atria, pulmonary veins, and systemic veins are all elevated to similar levels (i.e., within 5 mmHg of one another). Despite these hemodynamic changes, myocardial function may be normal or only slightly impaired in chronic constrictive pericarditis. However, the fibrotic process may extend into the myocardium and cause myocardial scarring and atrophy, and venous congestion may then be due to the combined effects of the pericardial and myocardial lesions.

In constrictive pericarditis, the right and left atrial pressure pulses display an M-shaped contour, with prominent x and y descents. The y descent, which is absent or diminished in cardiac tamponade, is the most prominent deflection in constrictive pericarditis; it reflects rapid early filling of the ventricles. The y descent is interrupted by a rapid rise in atrial pressure during early diastole, when ventricular filling is impeded by the constricting pericardium. These characteristic changes are transmitted to the jugular veins, where they may be recognized by inspection. In constrictive pericarditis, the ventricular pressure pulses in both ventricles exhibit characteristic “square root” signs during diastole. These hemodynamic changes, although characteristic, are not pathognomonic of constrictive pericarditis and may also be observed in cardiomyopathies characterized by restriction of ventricular filling (Chap. 21) (Table 22-2).


Weakness, fatigue, weight gain, increased abdominal girth, abdominal discomfort, a protuberant abdomen, and edema are common. The patient often appears chronically ill, and in advanced cases there are anasarca, skeletal muscle wasting, and cachexia. Exertional dyspnea is common, and orthopnea may occur, although it is usually not severe. Acute left ventricular failure (acute pulmonary edema) is very uncommon. The cervical veins are distended and may remain so even after intensive diuretic treatment, and venous pressure may fail to decline during inspiration (Kussmaul’s sign). The latter is common in chronic pericarditis but may also occur in tricuspid stenosis, right ventricular infarction, and restrictive cardiomyopathy.

The pulse pressure is normal or reduced. In about one-third of cases, a paradoxical pulse can be detected. Congestive hepatomegaly is pronounced and may impair hepatic function and cause jaundice; ascites is common and is usually more prominent than dependent edema. The apical pulse is reduced and may retract in systole (Broadbent’s sign). The heart sounds may be distant; an early third heart sound (i.e., a pericardial knock, occurring at the cardiac apex 0.09–0.12 s after aortic valve closure) is often conspicuous; it occurs with the abrupt cessation of ventricular filling. A systolic murmur of tricuspid regurgitation may be present.

The ECG frequently displays low voltage of the QRS complexes and diffuse flattening or inversion of the T waves. Atrial fibrillation is present in about one-third of patients. The chest roentgenogram shows a normal or slightly enlarged heart; pericardial calcification is most common in tuberculous pericarditis. Pericardial calcification may, however, occur in the absence of constriction.

Inasmuch as the usual physical signs of cardiac disease (murmurs, cardiac enlargement) may be inconspicuous or absent in chronic constrictive pericarditis, hepatic enlargement and dysfunction associated with jaundice and intractable ascites may lead to a mistaken diagnosis of hepatic cirrhosis. This error can be avoided if the neck veins are inspected carefully in patients with ascites and hepatomegaly. Given a clinical picture resembling hepatic cirrhosis, but with the added feature of distended neck veins, a careful search for thickening of the pericardium by imaging (see Fig. 12-6) should be carried out and may disclose this curable or remediable form of heart disease.

The transthoracic echocardiogram typically shows pericardial thickening, dilation of the inferior vena cava and hepatic veins, and a sharp halt in ventricular filling in early diastole, with normal ventricular systolic function and flattening of the left ventricular posterior wall. Atrial enlargement may be seen, especially in patients with long-standing constrictive physiology. There is a distinctive pattern of transvalvular flow velocity on Doppler flow-velocity echocardiography. During inspiration there is an exaggerated reduction in blood flow velocity in the pulmonary veins and across the mitral valve and a leftward shift of the ventricular septum; the opposite occurs during expiration. Diastolic flow velocity in the vena cavae into the right atrium and across the tricuspid valve increases in an exaggerated manner during inspiration and declines during expiration (Fig. 22-5). However, echocardiography cannot definitively exclude the diagnosis of constrictive pericarditis. MRI and CT scanning (Fig. 22-6) are more accurate than echocardiography in establishing or excluding the presence of a thickened pericardium. Pericardial thickening and even pericardial calcification, however, are not synonymous with constrictive pericarditis since they may occur without seriously impairing ventricular filling.



Cardiovascular magnetic resonance in a patient with constrictive pericarditis
. On the right is a basal short-axis view of the ventricles showing a thickened pericardium encasing the heart (arrows). On the left is a transaxial view, again showing the thickened pericardium, particularly over the right heart, but also a pleural effusion (Pl Eff). LV, left ventricle; RV, right ventricle. (From D Pennell: Cardiovascular Magnetic Resonance, in P Libby et al [eds]: Braunwald’s Heart Disease, 8th ed. Philadelphia, Elsevier, 2005.)


Like chronic constrictive pericarditis, cor pulmonale (Chap. 17) may be associated with severe systemic venous hypertension but little pulmonary congestion; the heart is usually not enlarged, and a paradoxical pulse may be present. However, in cor pulmonale, advanced parenchymal pulmonary disease is usually obvious and venous pressure falls during inspiration (i.e., Kussmaul’s sign is negative). Tricuspid stenosis (Chap. 20) may also simulate chronic constrictive pericarditis; congestive hepatomegaly, splenomegaly, ascites, and venous distention may be equally prominent. However, in tricuspid stenosis, a characteristic murmur as well as the murmur of accompanying mitral stenosis is usually present.

Because constrictive pericarditis can be corrected surgically, it is important to distinguish chronic constrictive pericarditis from restrictive cardiomyopathy (Chap. 21), which has a similar physiologic abnormality (i.e., restriction of ventricular filling). In many patients with restrictive cardiomyopathy the ventricular wall is thickened as shown on echocardiographic examination (Table 22-2). The features favoring the diagnosis of restrictive cardiomyopathy over chronic constrictive pericarditis include a well-defined apex beat, cardiac enlargement, and pronounced orthopnea with attacks of acute left ventricular failure, left ventricular hypertrophy, gallop sounds (in place of a pericardial knock), bundle branch block, and, in some cases, abnormal Q waves on the ECG. The typical echocardiographic features of constrictive pericarditis (see earlier) are useful in the differential diagnosis in chronic constrictive pericarditis (Fig. 22-5). CT imaging (usually with contrast) and MRI are key in distinguishing between restrictive cardiomyopathy and chronic constrictive pericarditis. In the former, the ventricular walls are hypertrophied, whereas in the latter, the pericardium is thickened and sometimes calcified. When a patient has progressive, disabling, and unresponsive congestive heart failure and displays any of the features of constrictive heart disease, Doppler echocardiography to record respiratory effects on transvalvular flow and an MRI or CT scan should be obtained to detect or exclude constrictive pericarditis, since the latter is usually curable.



Constrictive pericarditis.
 Doppler schema of respirophasic changes in mitral and tricuspid inflow. Reciprocal patterns of ventricular filling are assessed on pulsed Doppler examination of mitral valve (MV) and tricuspid valve (TV) inflow. (Courtesy of Bernard E. Bulwer, MD; with permission.)

TREATMENT Constrictive Pericarditis

Pericardial resection is the only definitive treatment of constrictive pericarditis and should be as complete as possible. Dietary sodium restriction and diuretics are useful during preoperative preparation. Coronary arteriography should be carried out preoperatively in patients older than 50 years of age to exclude unsuspected coronary artery disease. The benefits derived from cardiac decortication are usually progressive over a period of months. The risk of this operation depends on the extent of penetration of the myocardium by the fibrotic and calcific process, the severity of myocardial atrophy, the extent of secondary impairment of hepatic and/or renal function, and the patient’s general condition. Operative mortality is in the range of 5% to 10%; the patients with the most severe disease are at highest risk. Therefore, surgical treatment should, if possible, be carried out relatively early in the course.

Subacute effusive-constrictive pericarditis

This form of pericardial disease is characterized by the combination of a tense effusion in the pericardial space and constriction of the heart by thickened pericardium. It shares a number of features both with chronic pericardial effusion producing cardiac compression and with pericardial constriction. It may be caused by tuberculosis (see later), multiple attacks of acute idiopathic pericarditis, radiation, traumatic pericarditis, renal failure, scleroderma, and neoplasms. The heart is generally enlarged, and a paradoxical pulse and a prominent x descent (without a prominent y descent) are present in the atrial and jugular venous pressure pulses. After pericardiocentesis, the physiologic findings may change from those of cardiac tamponade to those of pericardial constriction. Furthermore, the intrapericardial pressure and the central venous pressure may decline, but not to normal. The diagnosis can be established by pericardiocentesis followed by pericardial biopsy. Wide excision of both the visceral and parietal pericardium is usually effective therapy.

Tuberculous pericardial disease

image This chronic infection is a common cause of chronic pericardial effusion, although less so in the United States than in Africa, Asia, the Middle East, and other parts of the developing world where active tuberculosis is endemic. The clinical picture is that of a chronic, systemic illness in a patient with pericardial effusion. It is important to consider this diagnosis in a patient with known tuberculosis, with HIV, and with fever, chest pain, weight loss, and enlargement of the cardiac silhouette of undetermined origin. If the etiology of chronic pericardial effusion remains obscure despite detailed analysis of the pericardial fluid (see earlier), a pericardial biopsy, preferably by a limited thoracotomy, should be performed. If definitive evidence is still lacking but the specimen shows granulomas with caseation, antituberculous chemotherapy is indicated.

If the biopsy specimen shows a thickened pericardium, pericardiectomy should be carried out to prevent the development of constriction. Tubercular cardiac constriction should be treated surgically while the patient is receiving antituberculous chemotherapy.


Pericardial cysts appear as rounded or lobulated deformities of the cardiac silhouette, most commonly at the right cardiophrenic angle. They do not cause symptoms, and their major clinical significance lies in the possibility of confusion with a tumor, ventricular aneurysm, or massive cardiomegaly. Tumors involving the pericardium are most commonly secondary to malignant neoplasms originating in or invading the mediastinum, including carcinoma of the bronchus and breast, lymphoma, and melanoma. The most common primary malignant tumor is the mesothelioma. The usual clinical picture of malignant pericardial tumor is an insidiously developing, often bloody pericardial effusion. Surgical exploration is required to establish a definitive diagnosis and to carry out definitive or, more commonly, palliative treatment.